Overexpression of myocilin in the Drosophila eye activates the unfolded protein response: implications for glaucoma.

ABSTRACT:

Background

Glaucoma is the world's second leading cause of bilateral blindness with progressive loss of vision due to retinal ganglion cell death. Myocilin has been associated with congenital glaucoma and 2-4% of primary open angle glaucoma (POAG) cases, but the pathogenic mechanisms remain largely unknown. Among several hypotheses, activation of the unfolded protein response (UPR) has emerged as a possible disease mechanism.

Methodology / principal findings

We used a transgenic Drosophila model to analyze whole-genome transcriptional profiles in flies that express human wild-type or mutant MYOC in their eyes. The transgenic flies display ocular fluid discharge, reflecting ocular hypertension, and a progressive decline in their behavioral responses to light. Transcriptional

SUBMITTER: Carbone MA 

PROVIDER: S-EPMC2615221 | biostudies-literature | 2009

REPOSITORIES: biostudies-literature